Pure red cell aplasia (PRCA) is a recognized but rare complication of systemic lupus erythematosus (SLE) and is characterized by the near absence of red blood cell precursors in the bone marrow but with normal megakaryocyte and granulocytes. We report a novel case of acquired PRCA occurring simultaneously with immune thrombocytopenia in the context of active SLE. Both syndromes were refractory to conventional treatment but responded to rituximab and cyclophosphamide.